BACKGROUND AND PURPOSE: Cough syncope typically occurs in patients with known chronic lung disease. The mechanism usually involves a combination of decreased venous return, increased cerebrospinal fluid pressure, and secondary hypocapnia, all resulting in cerebral arterial vasoconstriction. Cough syncope has not in the past been associated with occlusive cerebrovascular disease. CASE DESCRIPTION: We describe a 50-year-old man with a 6-month history of episodes of loss of consciousness during paroxysms of coughing. Physical examination showed asymmetrical upper extremity blood pressures and carotid and subclavian artery bruits. Pulmonary function studies were normal. Ultrasound and angiography showed total occlusion of the left common carotid artery, right internal carotid artery, and right vertebral artery; tight stenosis of the right subclavian artery; and a hypoplastic left vertebral artery. The patient had a left subclavian-to-left common carotid artery bypass and has had no syncope since that time. CONCLUSIONS: To our knowledge, this is the first reported case of cough syncope and severe cerebrovascular disease in which surgery led to amelioration of symptoms. Cerebrovascular occlusive disease may contribute to cough syncope.